Abstract: POEMS syndrome is a rare clonal plasma cell disease characterized by polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes. Significant advances have been made in the diagnosis and treatment of POEMS syndrome over the last decade. In this study, we reviewed the diagnostic criteria and characteristic features of POEMS syndrome. We also focused on the role of the characteristic features of POEMS syndrome in early diagnosis. Autologous peripheral blood stem cell transplantation has become the first-line treatment for younger patients with normal organ function because it has resulted in a high response rate and durable remission. Melphalan and dexamethasone is an effective and well-tolerated treatment for older patients and those with organ dysfunction. Patients with poor performance status or renal function can benefit from novel agents that can also improve transplantation eligibility.